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PACS gene family-related neurological diseases: limited genotypes and diverse phenotypes

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World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han zhang Kai Gao Shuang Wang yue-hua zhang Zhi-Xian Yang Ye Wu Yu-Wu Jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BeijingChina Children Epilepsy Center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina Center of Epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACS gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACS include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACS variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACS gene family-related neurological diseases were reported,of which 43 were PACS2-related cases and the remaining were PACSI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(ASMs).The most efficacious and frequently prescribed ASMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACSI ***203Trp and PACS2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACS heterozygous missense variants were *** pathogenic variant sites of PACSI and PACS2 were quite limited but located in different regions.A CNV destroying part of the PACS2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACS gene family.

关键词： Developmental delay Epilepsy Intellectual disability PACSI PACS2

Necroptosis contributes to non-alcoholic fatty liver disease pathoetiology with promising diagnostic and therapeutic functions

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World Journal of Gastroenterology 2024年第14期30卷 1968-1981页

作者： Hong-Ju Sun Bo Jiao Yan Wang yue-hua zhang Ge Chen Zi-Xuan Wang Hong Zhao Qing Xie Xiao-hua Song Department of General Medicine Qingdao Central HospitalUniversity of Health and Rehabilitation Sciences(Qingdao Central Medical Group)Qingdao 266042Shandong ProvinceChina Department of Gastroenterology Qingdao Central HospitalUniversity of Health and Rehabilitation Sciences(Qingdao Central Medical Group)Qingdao 266042Shandong ProvinceChina Department of Medical Administration Qingdao Central HospitalUniversity of Health and Rehabilitation Sciences(Qingdao Central Medical Group)Qingdao 266042Shandong ProvinceChina Qingdao Medical College Qingdao UniversityQingdao 266042Shandong ProvinceChina Department of Infectious Diseases Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China

Nonalcoholic fatty liver disease(NAFLD)is the most prevalent type of chronic liver ***,the disease is underappreciated as a remarkable chronic disorder as there are rare managing *** studies have focused on determining NAFLD-caused hepatocyte death to elucidate the disease pathoe-tiology and suggest functional therapeutic and diagnostic ***,ferroptosis,and necroptosis are the main subtypes of non-apoptotic regulated cell deaths(RCDs),each of which represents particular *** the complexity of the findings,the present study aimed to review these types of RCDs and their contribution to NAFLD progression,and subsequently discuss in detail the role of necroptosis in the pathoetiology,diagnosis,and treatment of the *** study revealed that necroptosis is involved in the occurrence of NAFLD and its progression towards steatohepatitis and cancer,hence it has potential in diagnostic and therapeutic ***,further studies are necessary.

关键词： Nonalcoholic fatty liver disease Apoptosis Necroptosis Cell death Diagnosis Treatment

Ruin Probabilities of a Surplus Process Described by PDMPs

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Acta Mathematicae Applicatae Sinica 2008年第1期24卷 117-128页

作者： Jing-min He Rong Wu hua-yue zhang Department of Mathematics and LPMC Nankai University Tianjin 300071 China

In this paper we mainly study the ruin probability of a surplus process described by a piecewise deterministic Markov process （PDMP）. An integro-differential equation for the ruin probability is derived. Under a certain assumption, it can be transformed into the ruin probability of a risk process whose premiums depend on the current reserves. Using the same argument as that in Asmussen and Nielsen, the ruin probability and its upper bounds are obtained. Finally, we give an analytic expression for ruin probability and its upper bounds when the claim-size is exponentially distributed.

关键词： Ruin probability piecewise deterministic Markov process integro-differential equation volterra equation

Prevention of malignant digestive system tumors should focus on the control of chronic inflammation

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World Journal of Gastrointestinal Oncology 2023年第3期15卷 389-404页

作者： yue-hua zhang Xiao-Lin Chen Yi-Ran Wang Yu-Wei Hou Yao-Dong zhang Kai-Juan Wang College of Public Health Zhengzhou UniversityZhengzhou 450001Henan ProvinceChina Henan International Joint Laboratory of Prevention and Treatment of Pediatric Diseases Henan Children's Hospital Zhengzhou Children’s HospitalChildren's Hospital Affiliated to Zhengzhou UniversityZhengzhou 450018Henan ProvinceChina Department of Prenatal Diagnosis Center The Third Affiliated Hospital of Zhengzhou UniversityZhengzhou 450052Henan ProvinceChina Henan Children’s Hospital Zhengzhou Children’s Hospital Children’s Hospital Affiliated to Zhengzhou UniversityKey Laboratory of Tumor Epidemiology of Henan ProvinceState Key Laboratory of Esophageal Cancer Prevention&TreatmentZhengzhou UniversityZhengzhou 450001Henan ProvinceChina

Chronic inflammation,through a variety of mechanisms,plays a key role in the occurrence and development of digestive system malignant tumors(DSMTs).In this study,we feature and provide a comprehensive understanding of DSMT prevention strategies based on preventing or controlling chronic *** development and evaluation of cancer prevention strategies is a longstanding *** prevention,especially in the early stage of life,should be emphasized throughout the whole life *** such as the time interval for colon cancer screening,the development of direct-acting antiviral drugs for liver cancer,and the Helicobacter pylori vaccine all need to be explored in long-term,large-scale experiments in the future.

关键词： Chronic inflammation Digestive system malignant tumors Prevention Screening Life course Gastrointestinal cancer

Microneedle patch loaded with ferritin-nanocaged doxorubicin for locally targeted drug delivery and efficient skin cancer treatment

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Particuology 2024年第5期88卷 282-289页

作者： Feng Li Yuan Yang hua yue Shuang Wang Xiao zhang Wei Wei State Key Laboratory of Biochemical Engineering Institute of Process EngineeringChinese Academy of SciencesBeijing100190China Key Laboratory of Biopharmaceutical Preparation and Delivery Chinese Academy of SciencesBeijing100190China School of Chemical Engineering University of Chinese Academy of SciencesBeijing100049China

Ferritin has emerged as a promising nanocarrier for delivering therapeutic agents to ***,the limited drug loading and the off-target impacts after systemic administration remain challenges for cancer treatment with ferritin-based ***,we develop a microneedle patch loaded with ferritin-nanocaged doxorubicin(DoxFe@Fn/MN)for skin cancer ***,doxorubicin(Dox)is encapsulated in ferritin(Fn)using an iron core-assisted strategy,which results in a 3.4-fold increase in Dox loading compared to the direct loading ***,a polyvinyl alcohol-based microneedle(MN)patch is used for the transdermal delivery of DoxFe@Fn,enabling targeted tumour accumulation of DoxFe@Fn and preventing off-target *** released DoxFe@Fn can bind to CD71 highly expressed on skin cancer cells,facilitating its *** a result,the DoxFe@Fn/MN therapy presents a robust antitumour effect in a melanoma tumour model,showing its potential as a promising therapeutic modality for skin cancer treatment.

关键词： Ferritin Microneedle Skin cancer Drug delivery

Clinical characteristics of two cohorts of infantile spasms: response to pyridoxine or topiramate monotherapy

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World Journal of Pediatrics 2018年第3期14卷 290-297页

作者： Jiao Xue Ping Qian Hui Li Ye Wu Hui Xiong yue-hua zhang Zhi-Xian Yang Department of Pediatrics Peking University First HospitalNo.1Xi'anmen StreetXicheng DistrictBeijing 100034China

Background Infantile spasms (IS) was an epileptic disease with varied treatment widely among clinicians. Here, we aimed to compare and analyze the clinical characteristics of IS response to pyridoxine or topiramate monotherapy (TPM control IS). Methods The clinical manifestations, treatment processes and outcomes were analyzed in 11 pyridoxine responsive IS and 17 TPM-control IS. Results Of the 11 patients with pyridoxine responsive IS, nine were cryptogenic/idiopathic. Age of seizure onset was 5.36 ± 1.48 months. Spasms were controlled within a week in most of the patients. At the last follow-up, EEG returned to normal in 8. Psychomotor development was normal in 6, mild delay in 3, severe delay in 2. Of the 17 patients with TPM-control IS, 10 were cryptogenic/idiopathic. The age of seizure onset was 5.58 ± 2.09 months. All patients were controlled within a month. At the last follow-up, EEG was normal in 10. Psychomotor development was normal in 8, mild delay in 5, severe delay in 4. Genetic analysis did not show any meaningful results. Conclusions The clinical characteristics and disease courses of pyridoxine responsive IS and TPM-control IS were similar, which possibly clued for a same pathogenic mechanism. Pyridoxine should be tried first in all IS patients, even in sympto-matic cases. If patients were not responsive to pyridoxine, TPM could be tried.

关键词： Infantile spasms Pyridoxine Topiramate

Elite Dung Beetle Optimization Algorithm for Multi-UAV Cooperative Search in Mountainous Environments

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Journal of Bionic Engineering 2024年第4期21卷 1677-1694页

作者： Xiaoyong zhang Wei yue The College of Marine Electrical Engineering Dalian Maritime UniversityDalian116026China

This paper aims to address the problem of multi-UAV cooperative search for multiple targets in a mountainous environment,considering the constraints of UAV dynamics and prior environmental ***,using the target probability distribution map,two strategies of information fusion and information diffusion are employed to solve the problem of environmental information inconsistency caused by different UAVs searching different areas,thereby improving the coordination of UAV ***,the task region is decomposed into several high-value sub-regions by using data clustering *** on this,a hierarchical search strategy is proposed,which allows precise or rough search in different probability areas by adjusting the altitude of the aircraft,thereby improving the search ***,the Elite Dung Beetle Optimization Algorithm(EDBOA)is proposed based on bionics by accurately simulating the social behavior of dung beetles to plan paths that satisfy the UAV dynamics constraints and adapt to the mountainous terrain,where the mountain is considered as an obstacle to be ***,the objective function for path optimization is formulated by considering factors such as coverage within the task region,smoothness of the search path,and path *** effectiveness and superiority of the proposed schemes are verified by the simulation.

关键词： Mountainous environment Multi-UAV cooperative search Environment information consistency Elite dung beetle optimization algorithm(EDBOA) Path planning

Yield, Leaf Senescence, and Cry1Ac Expression in Response to Removal of Early Fruiting Branches in Transgenic Bt Cotton

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Agricultural Sciences in China 2008年第6期7卷 692-702页

作者： DONG He-zhong TANG Wei LI Wei-jiang LI Zhen-huai NIU yue-hua zhang Dong-mei Cotton Research Center Shandong Academy of Agricultural Sciences/Shandong Provincial Key Lab for Cotton Culture and PhysiologyJinan 250100 P.R.China

Two-year field experiments were conducted at Linqing, Yellow River valley of China, to study the plant response to the removal of early fruiting branches in transgenic Bt （Bacillus thuringiensis） cotton （Gossypium hirsutum L.） from 2003 to 2004. Plants were undamaged and treated by removing two basal fruiting branches （FB） at squaring to form the control and the removal treatment, respectively. The plant height, leaf area （LA）, dry weight of fruiting forms （DWFF）, the number of fruiting nodes （NFN）, photosynthetic （Pn） rate, and levels of leaf chlorophyll （Chl）, N, P, K, and Cry lAc protein in main- stem leaves were measured at a 10- or 20-d interval after FB removal, and the sink/source ratio as indicated by NFN/LA and DWFF/LA was determined. FB removal significantly increased the plant height, LA, and plant biomass in both years. Lint yields were increased 7.5 and 5.2% by removal compared with their controls in 2003 and 2004, respectively. Significant increases in boll size （5.7 and 5.1%） were also observed in removal than in control for both years. Either NFN/LA or DWFF/LA was significantly reduced by removal before 40 d after removal; however, both NFN/LA and DWFF/LA were significantly enhanced by FB removal at 80 d after removal compared to the untreated control. There was no significant difference in fiber quality in the first two harvests between removal and control, but fiber strength and micronarie in the third harvest were significantly improved by FB removal. In terms of leaf Chl, Pn rate, levels of total N, P, and K in late season, leaf senescence was considerably delayed by FB removal. Levels of CrylAc protein in the fully expanded young leaves were considerably higher in FB-excised plants than in control, indicating FB removal enhanced CrylAc expression. It is suggested that the yield and quality improvement with FB removal may be attributed to the increased NFN/LA or DWFF/LA in late season and delayed leaf senescence, respectively. FB removal can be a

关键词： branch removal Bt cotton Cry lAc protein leaf senescence photosynthetic rate sink/source ratio

High Temperature Iron Ethylene Polymerization Catalysts Bearing N,N,N'-2-(1-(2,4-Dibenzhydryl-6-fluorophenylimino)ethyl)-6-(1-(arylphenylimino)ethyl)pyridines

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Chinese Journal of Polymer Science 2024年第2期42卷 188-201,I0007页

作者： Chantsalnyam Bariashir Qiu-yue zhang Bayasgalan Ulambayar Gregory A.Solan Tong-Ling Liang Wen-hua Sun Institute of Chemistry and Chemical Technology Mongolian Academy of SciencesUlaanbaatar13330Mongolia Key Laboratory of Engineering Plastics and Beijing National Laboratory for Molecular Science Institute of ChemistryChinese Academy of SciencesBeijing100190China Department of Chemistry University of LeicesterUniversity RoadLeicesterLE17RHUK State Key Laboratory for Oxo Synthesis and Selective Oxidation Lanzhou Institute of Chemical PhysicsChinese Academy of SciencesLanzhou730000China

The N,N,N'-ferrous chloride complexes,[2-{CMeN(2,4-(CHPh)_(2)-6-FC_(6)H_(2))}-6-(CMeNAr)C_(5)H_(3)N]FeCl_(2)(Ar=2,6-Me_(2)C_(6)H_(3) Fe1,2,6-Et_(2)C_(6)H_(3)Fe2,2,6-^(i)Pr_(2)C_(6)H_(3) Fe3,2,4,6-Me_(3)C_(6)H_(2) Fe4 and 2,6-Et_(2)-4-MeC_(6)H_(2) Fe5),each possessing one N-2,4-dibenzhydryl-6-fluorophenyl group,were readily synthesized from their respective unsymmetrical bis(imino)pyridines,*** identification of Fe2 highlighted the variation in the steric properties provided by the dissimilar N-aryl *** pre-treatment with either MAO or MMAO,complexes Fe1-Fe5 all displayed,at an operating temperature of 80℃,high activities for ethylene polymerization with levels falling in the order:Fe4> Fe1> Fe5> Fe2> ***,Fe4/MAO displayed the highest activity of 1.94×10^(7) g_(PE)·mol_(Fe)^(-1)·h^(-1) of the study with only a modest loss in performance at 90℃.Generally,the resulting polyethylenes were highly linear(T_(m) range:122-132℃),narrowly disperse and of low molecular weight(M_(w) range:6.73-46.04kg·mol^(-1)),with the most sterically hindered Fe3 forming the highest molecular weight polymer of the ***-group analysis by ^(1)H-and ^(13)CNMR spectroscopy revealed saturated alkyl(n-propyl and i-propyl) and unsaturated vinyl chain ends indicative of the role of both β-H elimination and chain transfer to aluminum as termination *** comparison with previou sly reported iron precatalysts with similar tridentate ligand skeletons,it is evident that the introduction of a large benzhydryl group in combination with a fluorine as the ortho-substituents of one N-aryl group has the effect of enhancing thermal stability of the iron polymerization catalyst whilst maintaining appreciable polymer molecular weight.

关键词： Iron catalyst Ethylene polymerization Linear polyethylene Fluoride effect Thermal stability